J Rheum Dis.  2012 Apr;19(2):104-107. 10.4078/jrd.2012.19.2.104.

A Cases of Adult Onset Still's Disease with Hemolytic Anemia

• Jung JK
• Kim YJ
• Byon CK
• Lee SY
• Lee SW
• Chung WT

• KMID: 2223109
• DOI: http://doi.org/10.4078/jrd.2012.19.2.104

Abstract

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology. AOSD is characterized by fever, arthralgia, salmon-colored skin rash, hepatosplenomegaly and its laboratory abnormalities include leukocytosis, elevated liver enzyme, negative autoantibody, and hyperferritinemia. The clinical course varied and severe complicated conditions, such as hemophagocytic syndrome, and disseminated intravascular coagulation, occurred occasionally. Such a complication is accompanied with hemolytic anemia and lead to be a fatal course. We report the first case of AOSD with hemolytic anemia, which improved with high dose steroid therapy.

Keyword

Adult onset still disease; Hemolytic anemia

MeSH Terms

Adult
Anemia, Hemolytic
Arthralgia
Disseminated Intravascular Coagulation
Exanthema
Fever
Humans
Leukocytosis
Liver
Lymphohistiocytosis, Hemophagocytic
Still's Disease, Adult-Onset

Figure

• 

  Figure 1 Peripheral blood shows polychromasia and schistocytes (Wright-Giemsa stain, ×1,000).

• 

  Figure 2 Bone marrow aspiration shows a slight increase in the number of myeloid precursors. The marrow is slightly hypocellular (Wright-Giemsa stain, ×200).

• 

  Figure 3 Abdomen CT shows hepatosplenomegaly.

Reference

1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992. 19:424–430.

2. Bywaters EG. Still's disease in the adult. Ann Rheum Dis. 1971. 30:121–133.

3. Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991. 70:118–136.

4. Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease. Ann Rheum Dis. 2006. 65:564–572.

5. Kumakura S, Ishikura H, Umegae N, Yamagata S, Kobayashi S. Autoimmune-associated hemophagocytic syndrome. Am J Med. 1997. 102:113–115.

6. Bagnari V, Colina M, Ciancio G, Govoni M, Trotta F. Adult-onset Still's disease. Rheumatol Int. 2010. 30:855–862.

7. Mert A, Ozaras R, Tabak F, Bilir M, Ozturk R, Ozdogan H, et al. Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. Clin Rheumatol. 2003. 22:89–93.

8. Wouters JM, van der Veen J, van de Putte LB, de Rooij DJ. Adult onset Still's disease and viral infections. Ann Rheum Dis. 1988. 47:764–767.

9. Omagari K, Matsunaga Y, Yamashita H, Nishiyama H, Hazama H, Oda H, et al. Successful treatment with cyclosporin in adult-onset Still disease manifesting as acute hepatitis with marked hyperferritinemia. Am J Med Sci. 2003. 326:148–151.

10. Kötter I, Wacker A, Koch S, Henes J, Richter C, Engel A, et al. Anakinra in patients with treatment-resistant adult-onset Still's disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum. 2007. 37:189–197.

11. van de Putte LB, Wouters JM. Adult-onset Still's disease. Baillieres Clin Rheumatol. 1991. 5:263–275.

12. Masson C, Le Loët X, Lioté F, Renou P, Dubost JJ, Boissier MC, et al. Adult Still's disease. Part II. Management, outcome, and prognostic factors. Rev Rhum Engl Ed. 1995. 62:758–765.

13. Coombs RRA, Mourant AE, Race RR. A new test for the detection of weak and incomplete Rh agglutinins. Br J Exp Pathol. 1945. 26:255–266.

14. Karasawa M. Autoimmune hemolytic anemia. Nihon Rinsho. 2008. 66:520–523.

15. Pirofsky B. Clinical aspects of autoimmune hemolytic anemia. Semin Hematol. 1976. 13:251–265.